Nutritional support for adult сystic fibrosis patients receiving targeted treatment
https://doi.org/10.18093/0869-0189-2024-34-2-295-299
Abstract
Cystic fibrosis (CF) is characterized by the development of a severe nutritional deficiency. A low BMI directly correlates with low lung function and requires active nutritional support. Pathogenetic (targeted) therapy aimed at restoring the chlorine channel function also leads to weight gain. The effects of CFTR modulators on extrapulmonary pathology in adult CF patients in Russia have been described very little.
Aim. To evaluate the sequential impact of two targeted drugs – the potentiator ivacaftor and the triple combination of CF transmembrane regulator modulators elexacaftor/tezacaftor/ivacaftor – on the nutritional status of an adult patient with cystic fibrosis receiving nutritional support.
Conclusion. Therapy with CFTR modulator in combination with sipping nutritional support promotes significant weight gain in adult CF patients. The triple combination of elexacaftor/tezacaftor/ivacaftor has a more active effect on nutritional status than ivacaftor alone. The targeted therapy requires supervision by a nutritionist.
About the Authors
N. A. Krylova
Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation
Russian Federation
Russian Federation
Natalia A. Krylova, Researcher, Cystic Fibrosis Department
Orekhovyy bul’var 28, Moscow, 115682
E. L. Amelina
Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation
Russian Federation
Russian Federation
Elena L. Amelina, Candidate of Medicine, Head of the Cystic Fibrosis Departmen
Orekhovyy bul’var 28, Moscow, 115682
S. A. Krasovsky
Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation;
State Budgetary Healthcare Institution “City Clinical Hospital named after D.D.Pletnev of the Department of Health of Moscow City”
Russian Federation
Russian Federation
Stanislav А. Krasovskiy, Candidate of Medicine, Senior Researcher, Laboratory of Cystic Fibrosis, Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation; Physician, State Budgetary Healthcare Institution “City Clinical Hospital named after D.D.Pletnev of the Department of Health of Moscow City”
Orekhovyy bul’var 28, Moscow, 115682,
ul. Odinnadtsataya Parkovaya 32, Moscow, 105077
G. L. Shumkova
Federal State Budgetary Institution “Pulmonology Scientific Research Institute” under Federal Medical and Biological Agency of Russian Federation
Russian Federation
Russian Federation
Galina L. Shumkova, Candidate of Medicine, Consultant Otorhinolaryngologist, Researcher, Cystic Fibrosis Laborator
Orekhovyy bul’var 28, Moscow, 115682
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For citations:
Krylova N.A., Amelina E.L., Krasovsky S.A., Shumkova G.L. Nutritional support for adult сystic fibrosis patients receiving targeted treatment. PULMONOLOGIYA. 2024;34(2):295-299. (In Russ.) https://doi.org/10.18093/0869-0189-2024-34-2-295-299
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